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https://www.readbyqxmd.com/read/28742875/the-significance-of-inadequate-transcranial-doppler-studies-in-children-with-sickle-cell-disease
#1
Simon Greenwood, Colin Deane, Oliver L Rees, Ben Freedman, Suresh Kumar, Naser Ben Ramadan, Sarah Wilkinson, Grant Marais, Julie Lord, Subarna Chakravorty, Susan E Height, Kate Gardner, David C Rees
Sickle cell disease (SCD) is a common cause of cerebrovascular disease in childhood. Primary stroke prevention is effective using transcranial Doppler (TCD) scans to measure intracranial blood velocities, and regular blood transfusions or hydroxycarbamide when these are abnormal. Inadequate TCD scans occur when it is not possible to measure velocities in all the main arteries. We have investigated the prevalence and significance of this in a retrospective audit of 3915 TCD scans in 1191 children, performed between 2008 and 2015...
2017: PloS One
https://www.readbyqxmd.com/read/28741138/trans-splenic-access-for-portal-venous-interventions-in-children-do-benefits-outweigh-risks
#2
Sheena Pimpalwar, Ponraj Chinnadurai, Alberto Hernandez, Kamlesh Kukreja, Shakeel Siddiqui, Henri Justino
BACKGROUND: The primary concern of trans-splenic access for portal interventions is the risk of life-threatening intraperitoneal bleeding. OBJECTIVE: To review the clinical indications and efficacy and evaluate the risk factors for intraperitoneal bleeding during trans-splenic portal interventions in children. MATERIALS AND METHODS: A retrospective review of consecutive patients who underwent trans-splenic portal interventions at a tertiary care pediatric institution between March 2011 and April 2017 was performed...
July 24, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28739553/acute-effects-of-blood-transfusion-on-insulin-sensitivity-and-beta-cell-function-in-children-with-beta-thalassemia-hbe-disease
#3
Somboon Wankanit, Ampaiwan Chuansumrit, Preamrudee Poomthavorn, Patcharin Khlairit, Sarunyu Pongratanakul, Pat Mahachoklertwattana
OBJECTIVE: To assess acute effects of blood transfusion on insulin sensitivity and β-cell function in thalassemia patients. METHODS: Fifty children and adolescents with β-thalassemia/HbE disease were enrolled in a prospective cohort study. Hemoglobin, serum ferritin and oral glucose tolerance test (OGTT) were performed prior to, and one week after blood transfusion. Insulin sensitivity indices [Homeostatic Model Assessment (HOMA) of Insulin Resistance (HOMA-IR), Whole Body Insulin Sensitivity Index (WBISI)] and β-cell function indices [HOMA of β-cell function (HOMA-β), Insulinogenic Index (IGI), and Disposition Index (DI)] were calculated from glucose and insulin levels obtained during the OGTT...
July 24, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/28731917/the-impact-of-iron-overload-in-acute-leukemia-chronic-inflammation-but-not-the-presence-of-nontransferrin-bound-iron-is-a-determinant-of-oxidative-stress
#4
Lale Olcay, Mustafa Serteser, Murat Kolay, Havva F Balci, Ülkü M Yildirim, Sibel A Tekgündüz, Tuncay Hazirolan, Yunus K Terzi
In the literature, studies on the oxidant effects of nontransferrin bound iron [NTBI (eLPI assay)] during chemotherapy of acute lymphoblastic leukemia and acute myeloblastic leukemia are lacking. We established NTBI and oxidative stress determinants (OSD), iron parameters, high-sensitive C-reactive protein (hs-CRP) levels, liver tests, cumulative chemotherapeutic doses, and transfused blood in 36 children with acute leukemia throughout chemotherapy. These parameters were determined at the beginning and end of chemotherapy blocks (11 time points) and in 20 healthy children using enzyme-linked immunosorbent assay, and colorimetric and fluorometric enzymatic methods...
July 20, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28731656/are-red-blood-cell-transfusions-associated-with-nosocomial-infections-in-critically-ill-children
#5
Omar E Naveda Romero, Andrea F Naveda Meléndez
Although the transfusionofblood products is common practice, its effects on the immune system have not been adequately studied. A prospective cohort study was conducted in critically ill children followed up until their death, transfer or discharge to establish an association between red blood cell transfusions (RBCTs) and nosocomial infections. A nosocomial infection was considered to be post-transfusional if it occurred within 14 days after RBCT. A total of 162 children were included in the study, 35 (21...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28728262/-evaluation-of-efficacy-of-immunosuppressive-therapy-plus-recombinant-human-thrombopoietin-for-children-with-severe-aplastic-anemia
#6
K Zhou, C X Liu, Y Li, J P Li, H H Fan, L Zhang, L P Jing, G X Peng, L Ye, Y Li, L Song, X Zhao, W R Yang, Z J Wu, F Chen, F K Zhang
Objective: To evaluate the therapeutic efficacy and safety of immunosuppressive therapy (IST) combined with recombinant human thrombopoietin (rhTPO) for severe aplastic anemia (SAA) in pediatric patients. Method: A retrospective case-control study was conducted and the clinical data of 45 pediatric patients with de novo SAA admitted to the Anemia Diagnosis and Treatment Center of Chinese Academy of Medical Sciences & Blood Disease Hospital during the period from December 2009 to December 2014 were analyzed...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28713150/a-jehovah-s-witness-adolescent-in-the-labor-and-delivery-unit-should-patient-and-parental-refusals-of-blood-transfusions-for-adolescents-be-honored
#7
Johan Christiaan Bester, Martin Smith, Cynthia Griggins
A 15-year-old was admitted to the labor and delivery unit for induction of a 41-week-gestation pregnancy. Her parents, members of Jehovah's Witnesses, and the patient, who had been studying the religion but had not yet been baptized, were adamant that no blood transfusions would be accepted even if a life-threatening hemorrhage were to occur. In our analysis, we examine the underlying ethical conflict and issues raised by this case. We considered two important ethical questions in analyzing the dilemma: first, whether adolescents are capable of providing autonomous and authentic refusals for lifesaving interventions; and second, whether parents can refuse such interventions for their adolescent children based on their religious beliefs...
2017: Narrative Inquiry in Bioethics
https://www.readbyqxmd.com/read/28709844/-transfusion-and-its-specific-problems-in-pediatrics-and-neonatology
#8
Y Pérel, C Runel, Y Huguenin, L Renesme, N Aladjidi
Principles of transfusion strategy have been used for neonates and children similar to adults. However, due to substantial discrepancies between physiology/pathology in children and in their adult counterparts, decisions, indications, and doses are different from those of adults, especially in neonates. Specific data and practice guidelines for blood product transfusion are reported owing to the experience of pediatrics and neonatology units and partners of the French Blood product bank.
July 11, 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28702679/predictive-factors-of-bleeding-among-pediatric-patients-undergoing-percutaneous-nephrolithotomy
#9
Cagri Senocak, Ridvan Ozbek, Omer Faruk Bozkurt, Ali Unsal
The purpose of this study is to determine the perioperative risk factors for increased blood loss in children undergoing percutaneous nephrolithotomy (PCNL).We retrospectively reviewed the data on pediatric patients who had undergone PCNL for stone disease in our department. Blood loss estimation was quantified by measuring the changes in hematocrit plus the volume of red blood cells transfused. Univariate and multivariate linear regression analyses were performed to evaluate risk factors associated with increased blood loss after pediatric PCNL...
July 12, 2017: Urolithiasis
https://www.readbyqxmd.com/read/28697170/critically-ill-children-with-hemophagocytic-lymphohistiocytosis-a-case-series-of-14-patients
#10
Esther Huimin Leow, Shui Yen Soh, Ah Moy Tan, Yee Hui Mok, Mei Yoke Chan, Jan Hau Lee
Children with hemophagocytic lymphohistiocytosis (HLH) are at an increased risk of critical illness. In this study, we described the clinical characteristics of critically ill children with HLH and identify factors associated with poor clinical outcomes. Children who were diagnosed with HLH with emergent admission to Children's Intensive Care Unit (CICU) between January 1, 2000 and October 31, 2015 were included. The primary outcome was CICU mortality. Over the 15-year period, there were 14 critically ill patients with HLH with 23 CICU admissions...
July 10, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28691229/desmopressin-use-for-minimising-perioperative-blood-transfusion
#11
REVIEW
Michael J Desborough, Kathryn Oakland, Charlotte Brierley, Sean Bennett, Carolyn Doree, Marialena Trivella, Sally Hopewell, Simon J Stanworth, Lise J Estcourt
BACKGROUND: Blood transfusion is administered during many types of surgery, but its efficacy and safety are increasingly questioned. Evaluation of the efficacy of agents, such as desmopressin (DDAVP; 1-deamino-8-D-arginine-vasopressin), that may reduce perioperative blood loss is needed. OBJECTIVES: To examine the evidence for the efficacy of DDAVP in reducing perioperative blood loss and the need for red cell transfusion in people who do not have inherited bleeding disorders...
July 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#12
REVIEW
Noemi Ba Roy, Patricia M Fortin, Katherine R Bull, Carolyn Doree, Marialena Trivella, Sally Hopewell, Lise J Estcourt
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD.Chronic kidney disease is defined as abnormalities of kidney structure or function, present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...
July 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28638157/complex-cardiac-surgery-on-patients-with-a-body-weight-of-less-than-5-kg-without-donor-blood-transfusion
#13
Wolfgang Boettcher, Frank Dehmel, Mathias Redlin, Oliver Miera, Michele Musci, Mi-Young Cho, Joachim Photiadis
Performing safe cardiac surgery in neonates or infants whose parents are Jehovah's Witnesses is only possible in a coordinated team approach. An unconditional prerequisite is a cardiopulmonary bypass (CPB) circuit with a very low priming volume to minimize hemodilution. In the past decade, we have developed a functional blood-sparing approach at our institution. The extracorporeal circuit was miniaturized. This had to be recently adapted, faced with a challenge associated with the switch to high-volume crystalloid cardioplegia...
June 2017: Journal of Extra-corporeal Technology
https://www.readbyqxmd.com/read/28635337/population-based-genetic-study-of-%C3%AE-thalassemia-mutations-in-mardan-division-khyber-pakhtunkhwa-province-pakistan
#14
Raj Muhammad, Muhammad Shakeel, Shoaib U Rehman, Muhammad A Lodhi
β-Thalassemia (β-thal) is the most prevalent hereditary blood disorder in Pakistan with a carrier rate of 5.0-8.0%. The homozygous affected children require frequent blood transfusions for their survival. This autosomal recessive disease can only be prevented through awareness programs, carrier screening, mutation detection, genetic counseling and prenatal diagnosis (PND). The present study aimed to determine the prevalence of various mutations causing β-thal and also to detect carriers of these mutations in families living in the Mardan Division, Khyber Pakhtunkhwa (KP) Province, Pakistan...
March 2017: Hemoglobin
https://www.readbyqxmd.com/read/28626543/cerebral-stroke-in-a-teenage-girl-with-paroxysmal-nocturnal-hemoglobinuria
#15
Francesco Gervasi, Lucia D'Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco, Piero Farruggia
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children's hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28582302/effect-of-erythropoietin-on-transfusion-requirements-for-craniosynostosis-surgery-in-children
#16
Hattan A Aljaaly, Salah A Aldekhayel, Julian Diaz-Abele, Mihiran Karunanayka, Mirko S Gilardino
BACKGROUND: Pediatric craniosynostosis surgery is associated with significant blood loss often requiring allogenic blood transfusion (ABT). This study explores the clinical effectiveness of preoperative erythropoietin (EPO) administration in pediatric craniosynostosis surgery in reducing transfusion requirements. METHODS: A systematic review and meta-analysis of the literature was performed for studies published in English language between 1946 and 2015. Inclusion criteria included original studies in the pediatric population (0-8 years of age) involving preoperative use of EPO in craniofacial procedures with quantitative reporting of perioperative blood transfusion...
June 2, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28578542/citrate-versus-heparin-anticoagulation-in-continuous-renal-replacement-therapy-in-small-children
#17
Paulien A M A Raymakers-Janssen, Marc Lilien, Ingrid A van Kessel, Esther S Veldhoen, Roelie M Wösten-van Asperen, Josephus P J van Gestel
BACKGROUND: Citrate is preferred over heparin as an anticoagulant in adult continuous renal replacement therapy (CRRT). However, its potential adverse effects and data on use in CRRT in infants and toddlers is limited. We conducted a prospective study on using citrate in CRRT in critically ill small children. METHODS: Children who underwent CRRT with the smallest filter in our PICU between November 2011 and November 2016 were included. Both heparin and citrate were applied according to a strict protocol...
June 4, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28576754/stroke-avoidance-for-children-in-rep%C3%A3%C2%BAblica-dominicana-sacred-protocol-for-a-prospective-study-of-stroke-risk-and-hydroxyurea-treatment-in-sickle-cell-anemia
#18
Neelum D Jeste, Luisanna M Sánchez, Gabriela S Urcuyo, Melissa E Bergés, Judy P Luden, Susan E Stuber, Teresa S Latham, Rafael Mena, Rosa M Nieves, Russell E Ware
BACKGROUND: In the Dominican Republic, where the burden of sickle cell anemia (SCA) is high, many children lack access to routine screening and preventative care. Children with SCA are at risk for stroke, an event that leads to significant morbidity and mortality. In the United States, screening via transcranial Doppler (TCD) identifies children with SCA at highest stroke risk, allowing early intervention with blood transfusions. The need for indefinite transfusions for primary stroke prevention limits their practicality in limited-resource countries...
June 2, 2017: JMIR Research Protocols
https://www.readbyqxmd.com/read/28575484/comprehensive-analysis-of-liberal-and-restrictive-transfusion-strategies-in-pediatric-intensive-care-unit
#19
Basak Akyildiz, Nazan Ulgen Tekerek, Ozge Pamukcu, Adem Dursun, Musa Karakukcu, Nazmi Narin, Mehmet Yay, Ferhan Elmali
Background: We prospectively compared restrictive and liberal transfusion strategies for critically ill children regarding hemodynamic and laboratory parameters. Methods: A total of 180 children requiring packed red blood cells (PRBCs) were randomized into two groups: the liberal transfusion strategy group (transfusion trigger < 10 g/dL, Group 1) and the restrictive transfusion strategy group (transfusion trigger ≤ 7 g/dL, Group 2). Basal variables including venous/arterial hemoglobin, hematocrit and lactate levels; stroke volume; and cardiac output were recorded at the beginning and end of the transfusion...
May 30, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28557286/incidence-and-predictors-of-massive-bleeding-in-children-undergoing-liver-transplantation-a-single-center-retrospective-analysis
#20
Benjamin Kloesel, Pete G Kovatsis, David Faraoni, Vanessa Young, Heung Bae Kim, Khashayar Vakili, Susan M Goobie
BACKGROUND: Liver transplantation represents a major surgery involving a highly vascular organ. Reports defining the scope of bleeding in pediatric liver transplants are few. AIMS: We conducted a retrospective analysis of liver transplants performed at our pediatric tertiary care center to quantify blood loss, blood product utilization, and to determine predictors for massive intraoperative bleeding. METHODS: Pediatric patients who underwent isolated liver transplantation at Boston Children's Hospital between 2011 and 2016 were included...
July 2017: Paediatric Anaesthesia
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